 Mofuchi syndrome is a rare genetic condition characterized by the development of multiple benign bone tumors called incondromas as well as soft tissue cavernous hemangiomas and an increased risk of malignant tumors. In this case, a patient was diagnosed with Mofuchi syndrome after being found to have a mutated form of the IDH1 gene, which is commonly associated with glioma development. This case highlights the importance of genetic testing in patients with Mofuchi syndrome who present with CNS tumors, as well as the need for further research into the relationship between IDH1 mutations and the development of gliomas in this population. This article was authored by Nurali Asharov, Iroda Mamadinova, Ados Moldebekov, and others.